Tell Your Story
Share Your Story and Raise Awareness
How did you become involved with VBF? Why do you feel it’s important to raise awareness for vascular birthmarks and the related syndromes? Please share your story with us. Please email your photos and story to VBF at firstname.lastname@example.org
When Aiden was born, he had a dark purple birthmark on the right side of his face. The attending pediatrician at the hospital told me that Aiden probably had what is called a port-wine stain (PWS), but could not give me a definite diagnosis or information.
After arriving home from the hospital, I did some extensive research on PWS. What I found was that this was not simply a birthmark or cosmetic issue, but a medical condition that would require early diagnosis, evaluation and treatment to prevent further complications. Some of the information I came across was devastating. I found that because Aiden’s PWS involves the first and second branch of the trigeminal nerve (the upper eyelid/forehead), Aiden's lesion was a marker for a rare condition called Sturge-Weber Syndrome (SWS) and that he would most likely develop glaucoma and seizures.
I immediately made an appointment for Aiden to see an ophthalmologist who confirmed that Aiden had a pressure of 40 (10 is normal) in his right eye (the eye affected by the PWS). Aiden was prescribed seven different eye drops and after they failed to lower the pressure, it was decided that Aiden needed a surgery called Trabeculotomy (performed at 2 ½ months of age in 2004). We made frequent post-op visits to make sure the surgery worked and we were happy to hear that the pressure in his right eye had dropped significantly. However, after an eye exam under anesthesia in 2006, we were told the pressure was increasing and Aiden was put back on eye drops. The drops are currently keeping his pressure at an acceptable level.
Aiden goes to the Beckman Laser Institute in Irvine approximately every eight weeks to receive laser treatments to lighten his port-wine stain. He began treatments when he was only four months old. He has had 15 treatments to date and the results are amazing!
Aiden was diagnosed at three months old as having Sturge-Weber Syndrome after having his first seizure. One night, Aiden was very fussy, which was not typical, and I noticed his left foot and hand twitching rhythmically. We rushed him to the emergency room where it was confirmed that Aiden was having focal (coming from one side of the brain) seizures. They ran CT, MRI and EEG tests and the result was positive for SWS. The right side of his brain has an overabundance of blood vessels just like in his skin. The excess blood vessels on the brain had caused calcification and atrophy (shrinking) which was causing the seizures. He had a couple of “stroke-like” seizures which caused him to have a weakening and partial paralysis on the left side of his body (called hemiparesis). We stayed in the hospital for two weeks trying to find a medication that would control his seizures. We were sent home on four different anti-epileptic drugs (Phenobarbital, Tegretol, Dilantin and Depakote). That combination worked for almost six months when the seizures returned and landed us back in the hospital. By that time, Aiden had been weaned off of all meds except Depakote. A new drug called Topamax was prescribed and after another two weeks in the hospital, we were able to go home.
Aiden made it almost two years seizure free until just before Christmas 2007, he started having brief focal and absence seizures again. This would happen up to four times a day. I called his neurologist right away and he increased Aiden’s dose of Topamax. After two days of the increased dose, the seizures stopped. I took Aiden in to see the neurologist for follow-up and he said that I should seriously consider a surgery called hemispherectomy because SWS is a progressive disease which means Aiden’s brain would continue to get worse over time. He gave me the number of a neurosurgeon at UCLA so we could take the next step in determining if Aiden would be a candidate for the surgery.
We went to UCLA for pre-surgical testing in April, 2008. They did a PET scan and extensive MRI, MRA, and MRV scan of Aiden's brain as outpatient procedures. Then, we were admitted for 7 days for EEG/Telemetry to map and record seizure activity. We then met with Dr. Mathern, the surgeon who would be performing the hemispherectomy. He said all the tests showed that there was only right-side brain involvement which made Aiden a candidate for the hemispherectomy. The procedure would involve removing the right hemisphere of Aiden’s brain and hopefully put an end to his seizures. He explained that the removal of the severely damaged brain tissue would better allow the healthy hemisphere to perform its duties. 80% of patients who undergo this surgery have seizure control with little to no medication.
The surgery was scheduled for August 21, 2008. That day was the hardest day of my life. I had to kiss my baby goodbye knowing that kiss might be the last one I would give him. He was in the O.R. for 12 long hours. Having my family there for support was crucial. To our amazement, Aiden was awake when we were finally allowed to see him in the ICU. The nurse told us he had been moving his arms and legs, which to me, was a miracle. The morning after surgery, I was sitting next to Aiden, waiting for him to wake up. When he did, I sang to him the song I’d always sing to him in the morning and he smiled at me and I knew at that moment everything was going to be alright.
Aiden recovered from his surgery so fast and since there were no resulting complications, he was allowed to go home after only seven days in the hospital. When he returned home, we continued to see progress and the best part, no seizures!
Aiden has had physical and occupational therapy since he was 5 months old because of severe developmental delays. He didn’t learn to crawl until he was almost 3 years old. He took his first steps when he turned 4. He can’t speak or chew food. For these issues, he has speech and feeding therapy. His developmental delays are believed to be caused by brain damage due to Sturge-Weber Syndrome and the seizures he had suffered. He continues to have therapy everyday and is making strides. He can now walk without assistance, is learning to feed himself and making more vocalizations. Although he is still behind developmentally, I feel that the hemispherectomy was the best choice for him. He is much more aware of the world around him and he is a happy, healthy boy. Aiden's future is a bright one and I know he will make a difference in this world.
It is an honor to be able to share Aiden’s story and our experience. It is gratifying and humbling to help people who are going through similar situations and facing decisions that will transform their lives.
-Rachel (Aiden's mom)
VBF has networked over 35,000 children and adults into treatment
Tyler Salvador and his twin sister, Shaine.